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With  the  Authors'  Compliments 

Pachymeningitis   Spinalis 
Externa. 

RECOVERY  COMPLETE. 


BY  F.  W.  LANGDON,  M.D. 
Professor  of  Nervous  and  Mental  Diseases,  Laura  Memorial  Woman's 
Medical  College;  Clinical  Professor  of  Nervous  Diseases,  Miami 
Medical  College ;  Neurologist  to  the  Cincinnati  Hospital. 

AND 

BY  ALBERT  H.  FREIBERG,  M.D. 

Professor  of   Surgery,  Laura  Memorial  Woman's  Medical  College; 

Orthopedic  Surgeon  to  the  Cincinnati  Hospital. 

CINCINNATI,  OHIO. 


REPRINTED  FROM 

THE  JOURNAL  OF  THE  AMERICAN  MEDICAL  ASSOCIATION 

AUGUST  26,  1899. 


CHICAGO 

American   Mkdical   Association   Press 
1899. 


Pachymeningitis   Spinalis 
Externa. 

RECOVERY  COMPLETE. 


BY  F.  W.  LANGDON,  M.D. 

Professor  of  Nervous  and  Mental*  Diseases,  Laura   Memorial  Woman's 

Medical  College;  Clinical  Professor  of  Nervous  Diseases,  Miami 

Medical  College ;  Neurologist  to  the  Cincinnati  Hospital. 


BY  ALBERT  H.  FREIBERG,  M.D. 

Professor  of   Surgery,  Laura  Memorial  Woman's  Medical  College; 
Orthopedic  Burgeon  to  the  Cincinnati  Hospital. 

CINCINNATI,  OHIO. 


REPRINTED  FROM 

THE  JOURNAL  OF  THE  AMERICAN  MEDICAL  ASSOCIATION 

AUGUST  26,  1899. 


CHICAGO 

Ameeican  Medical  Association  Pkess 
1899 


PACHYMENINGITIS  SPINALIS  EXTEENA. 

RECOVERY   COMPLETE. 


J.  X.,  aged  19,  an  American  of  Hebrew  parentage, 
single,  a  salesman  and  clerk,  was  referred  to  the  Cin- 
cinnati Hospital,  neurological  service,  by  Dr.  A.  V. 
Phelps,  February  28,  1898.1 

His  chief  complaint  was  "loss  of  power  in  arms  and 
legs"  of  six  weeks'  duration.  A  few  days  before  his  ad- 
mission he  developed  fever  and  a  feeling  of  general 
illness  which  led  him  to  apply  for  admission  to  the 
hospital. 

Family  History. — His  father  died  of  "throat  con- 
sumption," at  the  age  of  49,  having  been  ill  for  one 
year  following  a  wetting  and  severe  cold.  His  mother 
is  subject  to  acute  rheumatic  attacks.  One  brother  died 
in  infancy;  one  sister  has  inflammatory  rheumatism; 
three  brothers  and  one  sister  are  living  and  well. 

Personal  History. — He  has  been  generally  healthy 
and  remembers  no  illness  up  to  14  years  of  age,  when 
he  had  "sore  throat,"  lasting  two  days.  Syphilis  can 
positively  be  excluded.  He  had  gonorrhea  eight  months 
ago  followed  in  two  weeks. by  pain  and  stiffness  in 
the  back  of  the  neck,  which  he  called  "rheumatism." 
He  recovered  and  returned  to  his  business  within 
month,  and  was  Avell  for  the  succeeding  five  months  witli 
the  exception  of  occasional  pains  in  the  back  of  the  neck 
which  did  not  disable  him.  There  were  no  paralytic 
symptoms  during  this  time. 

The  onset  of  the  paralysis  was  gradual,  beginning 
about  six  weeks  before  his  admission  to  the  hospital 
and  being  accompanied  by  some  pain  and  rigidity  in  back 
of  the  neck.   Motor  weakness  began  in  the  left  arm,  and 

i  Acknowledgements  are  due  to  Drs.  Victor  Ray  and  John  S.  Rogtress. 
internes,  for  their  careful  examination  and  history  of  the  case,  of  which 
the  following-  is  an  abstract,  and  also  to  our  colleague,  Dr.  Herman  H, 
Hoppe,  for  kindly  permitting  the  use  of  notes  made  during  his  term  of 
service. 


306553 


gradually  increased  until  in  one  week  the  entire  limb 
was  helpless.  During  this  period  the  right  arm  became 
weak  and  he  gradually  lost  the  use  of  it.  The  legs 
were  affected  last ;  presumably  simultaneously.  Within 
a  month — probably  within  three  weeks — he  was  com- 
pletely disabled  in  all  four  extremities.  At  no  time- 
previous  to  admission  has  he  had  any  pain  in  his  limbs,. 


or  any  bladder  or  rectal  defect.  He  has  had  no  cough ;  has- 
had  some  headache  and  fever  for  two  or  three  days 
before  admission.  Temperature  shortly  after  admission 
was  101.6 — rising  to  104.2  within  twenty-four  hours — 
pulse  116,  respiration  30. 2 

2  This  pyrexia  and  its  accompaniments  are  apparently  due  to  an  inter- 
mittent tonsillitis,  so  far  as  can  be  seen  unconnected  with  his  spinal  lesion, 
and  subsiding  within  a  few  days.    See  temperature  chart  appended. 


Present  State —February  29,  1899.  Height  is  5  feet, 
8  inches,  weight  138,  of  medium  build,  dark  complexion; 
black  curly  hair,  dark  eyes. 

His  general  nutrition  is  good.  Part  of  the  thorax 
and  abdomen  is  covered  with  a  growth  of  pityriasis 
versicolor,  which  patient  states  has  been  present  for 
six  years.  His  neck  is  rigid,  its  tissues  apparently  in- 
filtrated and  indurated  posteriorly.  There  is  not  much 
tenderness  on  pressure  and  manipulation.  The  left 
tonsil  is  swollen,  its  follicles  being  distended  and  filled 
with  grayish-white  secretion.  The  uvula  and  soft  palate 
are  bifid;  no  edema  of  larynx  or  pharynx.  Patient  can 
swallow  and  talk  without  much  discomfort. 

His  mental  condition  is  good;  speech  not  impaired: 
cranial  nerves  not  affected. 

Trunk  and  Extremities. — Patient  can  not  stand  nor 
walk;  quadruplegia  is  present,  practically  complete  be- 
low elbow  and  knees;  he  can  flex  all  fingers  feebly.  The 
paralysis  is  moderately  rigid,  almost  "waxy"  in  type. 
Foot  and  wrist  drop  are  marked  on  both  sides.  He  can 
flex  and  extend  both  elbows  feebly.  Extension  at  elbows 
is  notably  stronger  than  'flexion.  Grasping  power  to 
dynamometric  test  E,  0.  L,  0. 

Sensation. — Tactile  sensibility  is  somewhat  dimin- 
ished in  acuity  at  the  ends  of  the  fingers,  elsewhere  ap- 
parently normal.  Pain  and  temperature  senses  are  not 
accurately  tested  at  this  date.  Note  change  in  cutaneous 
sensibility  four  dsys  later,  as  shown  by  charts  appended. 

Reflexes. — Organic.  No  defects  of  deglutition,  defeca- 
tion or  micturition.  Tendon:  Elbow-jerks  present  and 
equal;  wrist-jerks  present,  active  and  equal.  Knee-jerks 
present,  exaggerated  and  equal;  rectus  and  ankle-clonus 
present  and  equal  on  both  sides.  Cutaneous :  Not  ob- 
served. Vasomotor  system :  Patient  sweating  freely. 
Trophic:  No  muscular  atrophy  observable  to  ordinary 
examination.    No  trophic  ulcerations. 

Urine. — Eeaction  acid,  barely;  sp.  g.  1030;  phos- 
phates in  excess ;  albumin  and  sugar  absent. 

Blood-count  shows  a  moderate  leucocytosis  (16,500.) 
This  was  probably  due  to  the  intercurrent  tonsillitis. 

July  10. — A  tuberculin  test,  with  m.  xv  of  a  1-250  so- 
lution was  followed  in  two  hours  by  headache,  and  chill 


and  sweating  in  twenty-six  hours-  Two  days  after  ad- 
mission the  acnte  tonsillar  inflammation  subsided,  the 
temperature  dropped  to  normal,  and  for  the  next  four 
weeks  fluctuated  between  98  (a.  m.)  and  100  (p.  m.), 
only  once  during  this  time  rising  2  degrees  above  the 
'100  mark.  After  the  two  weeks  following  this  period, 
the  temperature  varied  between  98.4  (morning)  and 
99. -L-  (evening).     (See  chart  appended.) 


Be-examination  four  and  five  days  after  admission. 
(See  chart  No.  1.) 

Motion. — Quadruplegia,  of  waxy,  rigid  type  is  still 
present.  The  pectorals,  deltoids,  supinators,  small 
thenar  muscles,  and  short  extensors  of  toes  seem  abso- 
lutely powerless  on  both  sides.  Elsewhere  the  muscular 
power  is  barely  sufficient  to  flex  and  extend  joints,  ex- 


tension  seeming  rather  stronger  than  flexion  at  the 
elbows. 

The  tongue  protrudes  in  the  median  line,  is  longitud- 
inally fissured,  and  a  general  fibrillary  tremor  of  the  en- 
tire organ  is  present. 

Sensory  defects  of  a  "dissociation  type"  have  appeared 
as  per  Chart  No.  2.  These  consist  practically  of  diminu- 
tion and  loss  of  appreciation  of  heat  and — to  a  less  de- 
gree— of  cold,  with  preservation  of  tact  and  pain  over 
thorax,  abdomen  and  upper  arm  anteriorly,  and  on  fore- 
arm and  hands  anteriorly  and  posteriorly.  (See  Chart 
2.)  Tests  were  made  in  the  ordinary  manner  with 
test-tubes  of  decided  warm  and  cold  water  for  tempera- 
ture, cotton  and  pin  for  tact  and  pain.  A  week  later 
(see  Chart  3)  these  sensory  defects  were  increased  by 
addition  of  an  area  of  analgesia  over  the  thorax  anter- 
iorly. At  this  date  power  in  legs  has  apparently  in- 
creased, so  that  he  can  move  both  feet  and  legs  with 
considerable  freedom  as  he  lies  in  bed. 

Reflexes. — Pupils  are  moderately  dilated  when  at  rest, 
respond  well  to  accommodation  and  contract  to  light, 
but  do  not  dilate  farther  when  light  is  excluded.  Or- 
ganic :  Has  to  be  catheterized  for  a  day  or  two.  Vaso- 
motor: "Well  marked  "tache"  over  thorax  and  abdomen. 
Trophic :  jSTo  "bedsores"  or  other  ulceration. 

March  17. — Electrical  tests  now  and  later  showed 
partial  E.  D.  in  muscles  of  hypothenar  group  of  right 
hand,  as  evidenced  by  very  sluggish  contraction  to  gal- 
vanism and  nearly  equal  responses  to  both  poles,  though 
K.  C.  is  slightly  greater  than  A.  C. 

Muscles  elsewhere  react  normally  to  galvanic  and 
faradic  currents.  There  is  slight  improvement  in  power 
of  legs  and  arms. 

Twelve  days  later  the  motor  symptoms  were  practi- 
cally unchanged,  the  defects  of  heat  and  cold  sense  were 
somewhat  diminished  in  area,  but  persisted  on  thorax 
and  a  longitudinal  strip  along  the  inner  surface  of  the 
right  arm;  forearm  and  hand;  also  over  left  forearm 
and  hand  posteriorly.     (See  Chart  4.) 

March  29. — About  this  time  I  asked  my  colleague, 
Dr.  Freiberg,  orthopedic  surgeon  to  the  hospital,  to  see 
the  patient,  and  it  was  decided  by  his  advice  to  make 
extension  on  the  entire  vertebral  column  in  the  hope 


of  relieving  the  pressure  which  was  presumed  to  exist 
on  the  upper  cervical  cord.  The  effect  of  the  extension 
and  counterextension  on  the  sensory  symptoms  was 
startling.  Twenty-four  Lours  after  application  of  the 
apparatus,  it  was  difficult  to  detect  any  sensory  loss 
over  thorax  or  abdomen,  and  when  found  it  was  in  such 


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irregularly  scattered  patches  as  to  make  its  accurate  chart- 
ing impracticable.  By  April  10 — twelve  days  after  ap- 
plication of  extension — the  only  remaining  defects  were 
a  loss  of  temperature — heat  and  cold — sense  over  right 
hand,  palmar  and  dorsal  surfaces;  and  over  the  left 
hand,  dorsal  surface  only.  The  case  was  now  trans- 
ferred to  the  care  of  Dr.  Freiberg,  who  furnishes  the 
following : 


SURGICAL    HISTOEY. 

On  March  2S  I  examined  the  patient  whose  condition 
has  been  accurately  described  by  Dr.  Langdon.  I  found 
present,  exclusive  of  the  nerve  symptoms  already  de- 
scribed, a  considerable,  firm,  diffuse  swelling  immediately 
below  the  occiput  and  extending  downward  to  the  fourth 
cervical  spine.  Without  any  distinct  boss  it  was  suffi- 
cient to  render  indistinct  to  the  touch  the  vertebral  spine. 
There  was  considerable  tenderness  on  pressure  com- 
juained  of  in  tin.:  whole  swollen  area. 

There  was  no  torticollis  whatever,  but  the  patient  was 
unable  to  rotate  the  head  with  freedom.  Likewise  it  was 
impossible  for  him  to  bend  the  cervical  spine  backward 
to  the  normal  e  aont.  There  was  some  interference  with 
the  power  of  approximating  the  head  toward  either 
shoulder.  The  nodding  motion  was  not  interfered  with. 
Examination  of  the  pharynx  failed  to  show  anything 
abnormal. 

The  diagnosis  of  tubercular  disease  of  the  upper  cer- 
vical spine  was  made  with  some  reserve,  especially  with 
regard  to  its  exact  localization.  The  treatment  con- 
sisted in  the  application  of  weight  and  pulley  extension 
to  the  head,  the  weight  of  the  body  serving  as  counter- 
extension.  The  weights  were  increased  gradually  from 
three  to  twelve  pounds. 

The  change  in  the  patient's  condition  has  been  spoken 
of  by  Dr.  Langdon  as  startling;  this  is  by  no  means  an 
exaggeration.  Tbe  improvement  was,  however,  steadily 
progressive  from  this  time  forward. 

June  4. — The  appetite  is  good;  no  fever;  no  pain; 
less  rigidity.  He  can  move  his  neck  with  considerable 
freedom;  also  all  joints  of  extremities.  Grasp:  dyna- 
mometer E.  50,  L.  46;  knee-jerks  exaggerated;  E.  and 
L.  ankle-clonus  present  E.  and  L. 

The  patient  was  kept  in  bed  with  the  same  weight  at- 
tached until  Aug.  4,  1898.  At  this  time  an  examination 
showed  an  apparently  complete  return  to  the  normal 
in  every  regard  save  one — the  power  to  rotate  the  head 
laterally.  The  extension  was  therefore  removed  and  a 
Sayre  jury  mast  applied.  On  August  22  the  patient  ex- 
pressed a  desire  to  leave  the  hospital  and  was  permitted 
to  do  so,  wearing  no  apparatus,  and  he  walked  out  of  the 


10 


hospital  without  assistance.  About  six  weeks  ago  I  had 
an  opportunity  of  examining  the  patient,  and  his  con- 
dition remains  1he  same.  A  very  slight  interference 
with  rotation  of  the  head  and  the  induration  about  the 
upper  spinous  process,  which  has  never  disappeared  en- 
tirely, are  all  that  remained  of  the  conditions  found  upon 
the  first  physical  examination  of  the  neck.    The  patient 


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asserted  that  his  muscular  power  had  returned  in  full 
degree. 

ADDITIONAL  COMMENT  AND  SUMMAET. 

Since  leaving  the  hospital  the  patient  has  been  under 
our  separate  and  joint  observation  at  intervals  of  a  few 
weeks.  His  health  remains  good  and  he  attends  reg- 
ularly to  his  duties  as  salesman  and  clerk  in  a  store. 


Jl 

Neurologic  examination  May  19,  1899,  by  Dr.  Lang- 
don:  Height,  5  ft.  8  inches;  weight,  138  pounds;  gen- 
eral nutrition  good. 

Temperature,  99.2,  after  dinner  and  active  exercise; 
pulse  72,  regular.  A  slight  fullness  is  apparent  about 
the  fourth  or  fifth  cervical  spines.  Head  movements 
are  free  as  regards  rotation  and  extension;  somewhat 
limited  as  to  flexion  forward — can  not  touch  manubrium 
sterni  with  chin.  There  is  no  muscular  atrophy ;  no  de- 
fect of  gait  or  station.  Power  is  good  in  all  extremities. 
Grasp :  dynamometer  R.  105,  L.  98 — normal  average 
abor,t  80.  The  same  dynamometer  was  used  as  in  former 
tests. 

Sensation. — No  subjective  sensory  symptoms;  no  de- 
fect of  tact,  pain,  temperature  or  muscle  sense. 

Reflexes. — Organic :  No  bladder  or  rectal  defect,  no 
dysphagia.  Myotatic :  Elbow  and  wrist- jerks  present ;  right 
rather  more  active  than  left;  knee-jerks  somewhat  hy- 
peractive and  equal.  Eectus-clonus  and  ankle-clonus 
absent.  Cutaneous :  Palmar  absent ;  epigastric,  hypochon- 
driac, abdominal  and  cremasteric  present ;  normal.  Plan- 
tar present,  giving  marked  "flexor3"  responses  in  both 
feet. 

Diagnosis :  Having  in  view  the  quadruplegic  type  of 
paralysis,  the  possibilities  to  be  considered  were :  1, 
cerebral  diplegia;  2,  poliomyelitis  anterior;  3,  multiple 
neuritis;  4,  cervical  myelitis;  5,  spinal  tumor;  6,  hem- 
atomyelia;  7,  hematorrhachis,  external  or  internal;  8, 
leptomeningitis  spinalis ;  9,  pachymeningitis  spinalis 
interna;  10,  pachymeningitis  spinalis  externa. — pri- 
mary, a,  rheumatic;  b,  gonorrheal;  secondary  c,  tuber- 
cular (vertebral  caries.) 

Cerebral  diplegia  was  readily  ruled  out  by  the  history, 
the  gradual  onset,  and  absence  of  mental,  aphasic,  or 
cranial  nerve  symptoms.  Poliomyelitis  is  excluded  by 
the  rigid  type  of  paralysis  and  the  presence  of  marked 
sensory  defects.  Multiple  neuritis  was  not  indicated  by 
the  history,  and  was  further  eliminated  by  the  absence 
of  pain,  tenderness  and  muscular  atrophy.  Cervical 
transverse  myelitis  would  have  presented  sphincter  de- 
fects and  trophic  lesions,  which  were  absent.     Spinal 

3  See  Collier,  "Brain,"  1899. 


12 

tumor  is  conspicuous  for  the  presence  of  root  pains, 
which  were  absent.  Gumma  was  excluded  by  lack  of 
evidence  of  syphilis;  and  as  already  stated,  syphilis  is 
absolutely  excluded  in  the  case  by  recent  developments 
of  a  most  convincing  nature. 


Chart   5,  J.X.     Pachymeningitis   Spinalis   Externa. 

Hematomyelia  and  hematorrhaehis  have  a  sudden 
onset  with  rapid  improvement  if  the  patient  lives.  Lep- 
tomeningitis was  contraindicated  by  slow  onset,  the  ab- 


13 

senee  of  pain  or  hyperesthesia  on  movement  of  the  spine. 
the  evident  localized  chaiacter  of  the  lesion,  with  little 
tendency  to  spread.  Pachymeningitis  interna  was  ex- 
cluded by  absence  of  adequate  causes,  as  syphilis,  al- 
coholism and  trauma;  also  by  absence  of  marked  pain, 
and  irritative  root  symptoms,  as  well  as  absence  of  the 
later  muscular  atrophy.  Thus,  by  a  process  of  exclusion 
a  tentative  diagnosis  of  pachymeningitis  externa  was 
reached,  but  whether  this  was  primar}r,  i.  e.,  rheumatic 
or  gonorrheal,  as  the  history  might  suggest,  or  secondary 
to  vertebral  caries,  as  the  heredity  and  part  of  the 
symptomatology  would  indicate,  is  perhaps  an  open 
question. 

As  Dr.  Freiberg  has  stated,  the  diagnosis  of  tubercular 
disease  of  the  vertebra  was  made  with  some  reserve  by 
both  of  us.  Gowers4  states  on  this  point:  "When  clear 
indications  of  c?ries  precede  the  paralysis  the  nature 
of  the  ease  can  hardly  be  mistaken  .  .  .  When  the 
two  develop  together  mistakes  are  often  made,  but  are 
usually  due  to  the  want  of  repeated  examination  of  the 
spinal  column.  It  is  when  the  root  or  cord  symptoms 
precede  distinct  evidence  of  bone  disease  and  when  the 
latter  is  so  slight  as  to  be  equivocal  that  the  chief  real 
difficulty  in  diagnosis  occurs."  It  will  be  seen  that  the 
case  here  recorded  comes  under  the  third  condition  de- 
scribed above. 

To  sum  up :  The  symptoms  indicated  an  increasing 
pressure  of  exudate  with  its  incidence  at  the  first  and 
second  cervical  segments  of  the  cord  anteriorly,  thus 
compressing  the  ascending  anterolateral  tract  of  Gowers 
— temperature  sense  symptoms — and  the  pyramidal 
tracts  before  their  complete  passage  into  the  lateral 
columns.  In  no  other  situation  is  it  possible  to  conceive 
an  external  lesion  causing  the  motor  symptoms  here  pre- 
sented, viz. :  quadruplegia  with  waxy  rigidity  and  with- 
out muscular  atrophy  of  the  arms.  The  slight  electric 
changes  noticed  in  the  hypothenar  group  of  right  hand 
were  doubtless  due  to  a  nerve-root  involvement  lower 
down;  as  was  the  absence  of  pupilary  dilatation  on  re- 
moval of  light. 

*  Manual  of  Diseases  of  Nervous  System,  vol.  i,  p.  251. 


14 


Against  the  presence  of  primary  vertebral  disease  are 
the  supposed  "rheumatic"  constitution,  the  preceding 
gonorrheal  infection,  the  absence  of  trauma.  In  favor 
of  primary  vertebral  disease  of  tubercular  origin  are  the 
hereditary  factor,  the  tenderness  on  pressure,  with  thick- 


ening over  cervical  spine ;  the  reaction  to  the  tuberculin 
test,  which,  however,  was  much  delayed ;  and  finally  the 
doctrine  of  probabilities,  as  well  as  the  favorable  out- 
come. 


15 

Treatment. — In  addition  to  the  surgical  treatment 
proper,  of  extension  and  counterextension,  potassium 
iodid  was  given  in  twenty-grain  doses  for  aboiit  one 
month  following  his  admission  to  the  hospital.  Cod- 
liver  oil  and  hypophosphites  were  also  administered,  and 
the  patient  was  kept  in  the  open  air  Avhenever  practi- 
cable. 


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